The ichthyoses are a heterogeneous group of disorders with both inherited and acquired forms. Autosomal recessive congenital ichthyosis (ARCI) is a heterogeneous group of disorders that present at birth with the generalized involvement of skin without other systemic manifestations. Lamellar itchthyosis (LI) is a nonsyndromic itchthyosis, which comes under the umbrella of ARCI. Little is only known about the oral manifestations of this disorder. We report a case of LI with oral manifestations.

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Mastocytosis is a sporadic disease characterized by an abnormal accumulation of mast cells (MCs) in single or multiple organs. A 5-year-old girl who was diagnosed as cutaneous mastocytosis by our Dermatology Department when she was 4 months of age was admitted to our Pediatric Hematology Department by hepatosplenomegaly. Diagnosis of systemic mastocytosis (SM) was verified by one major and one minor WHO criteria: presence of multifocal, dense aggregates of MCs in bone marrow (major criteria) and confirmed by expression of CD2, CD25, and CD117 in bone marrow (minor criteria). During follow-up, systemic anaphylaxis was determined and treated for 3 times. We here present the first report of use of interferon-α-2a, methylprednisolone, and montelukast in life-threatening SM to achieve remission, suggesting a new option for treatment of SM in childhood.

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Harlequin ichthyosis (HI) is the most severe type of congenital ichthyosis, and it is extremely rare. It is inherited in an autosomal recessive fashion. Although previously thought to be lethal, recently there have been increased reports of prolonged survival, following improved supportive care and judicious use of systemic retinoids. We report a new case of HI in an infant born of a consanguineous marraige who succumbed on the 5^th day of birth despite intensive supportive care, a short review of literature regarding the condition is also presented.

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Onychomycosis refers to the nondermatophytic and dermatophytic involvement of nails. Literature suggests its increasing prevalence from the younger to the older age group. In addition to the important clinical consequences that the infection holds, it also acts as a reservoir of infection. We, hereby, report a case of a 3-year-old immunocompetent child with single nail onychomycosis due to Aspergillus niger. To the best of our knowledge, this is the youngest reported case of onychomycosis due to A. niger.

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Introduction: Skin diseases are a major health problem in the pediatric age group. Aim: To determine the prevalence and clinical characteristics of different pediatric dermatoses in a tertiary care centre in Kerala. Materials and Methods: Children with age 18 years and below with clinical evidence of cutaneous disorders were studied. Results: 500 cases were studied which showed a female preponderance of 51.4%. The most common dermatoses was infections and infestations (33.8%) followed by eczemas (32.6%), disorders of sweat and sebaceous glands (7.4%), keratinisation and paulosquamous disorders (4%). Nutritional disorders were seen in 1% of children. 3.6% had photodermatoses, 2.6% had hair and nail disorders and 0.4 % had adverse cutaneous drug reactions. Conclusions: Fungal infection was the most common infection noted in the study, followed by viral and bacterial infection. Allergic contact dermatitis was the commonest exogenous eczema and juvenile plantar dermatosis was the commonest endogenous eczemas. Acne, insect bite reaction and miliaria were the other common dermatoses.

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Aims and Objectives: To study the clinical features of herpes zoster (HZ) in childhood along with prevalence of human immunodeficiency virus (HIV) seropositivity. Materials and Methods: The study was carried out in the Department of Dermatology at a Tertiary Care Centre of Gujarat, India, for 6 years. Children aged ≤12 years with a diagnosis of HZ seen in the Departments of Dermatology were enrolled in a predesigned pro forma. Diagnosis of HZ was made on clinical grounds, confirmed by tzanck smear as and when required. Sera of all cases were tested for HIV. Results: Total of 34 children aged ≤12 years were enrolled in the study. Nineteen (55.88%) were boys and 15 (44.12%) were girls. The mean age was 9.26 years. In 97.06% patient have localized dermatomal involvement. Most common symptom was burning pain seen in 30 (88.24%) patients. Previous history of chickenpox was present in 19 (55.88%) patients. Evidence of immunosuppression on history, clinical examination, and investigations was present only in one patient, who had HIV infection. Conclusion: Although there is increased incidence of HZ in childhood, atypical presentations are rare, multidermatomal involvement is not commonly seen. Majority of these children do not show immunosuppression. Hence, we conclude HZ in childhood occurs as a relatively mild and self-limiting disease.

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Topical corticosteroids are one of the most frequently prescribed medications for skin diseases especially for atopic dermatitis (AD). Due to the rampant over-the-counter availability of these medications, misuse, and abuse of these drugs is well documented. The undesirable side-effects of corticosteroids are often seen in these patients. This has recently given rise to the phenomenon of“topical steroid phobia” where the patients or their caregivers are reluctant to use them even when prescribed by the physicians. This makes the proper management of steroid-responsive diseases like AD, very difficult. In this article, we have discussed the causes of emergence of this phobia, the problems it is posing and how to deal with them.

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Erythrokeratodermas are a rare group of genetic disorders presenting early in childhood as erythematous, hyperkeratotic plaques on the skin. There may be associated neurological involvement. A 12-year-old boy presented with symmetric keratotic plaques on the body since infancy. Psoriasis and pityriasis rubra pilaris were ruled out after histopathological examination of the skin. We are reporting a case of progressive symmetric erythrokeratoderma with classical lesions but can be missed due to unawareness.

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Epidermodysplasia verruciformis (EV) is a rare genetic disease caused by human papilloma virus, presenting with pityriasis versicolor-like, verruca-like, and seborrheic keratosis-like plaques and may predispose to cutaneous malignancy. We report two siblings with EV whose lesions regressed with systemic retinoids.

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Nevus comedonicus (NC) is a type of an epidermal nevus which was first described in 1895 by Kofmann. Clinically, it is characterized by linear lesion comprising numerous dilated follicular openings with keratinous plugs resembling classical comedones, seen mainly on the head and neck area followed by trunk and upper arm. Treatment options of NC include topical therapy, laser, and surgery. NC associated with epidermoid cyst is rare. We report here a case of NC affecting middle part of the neck over left sternocleidomastoid muscle with epidermoid cysts which was surgically treated.

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A 12-month-old boy presented with swelling all over body, reddish brown lesions over both lower extremities, and mild fever. On examination, pallor was present, vitals were stable, and child appeared well-except for edema and rashes. Pitting edema was present over all extremities and periorbital region. There was no edema over scrotum and abdominal wall. Skin examination showed erythematous, palpable, purpuric lesions (0.5–3 cm in diameter) over buttocks and lower limbs sparing trunk, face, and upper limbs. All other systems were within normal limits. Skin biopsy showed the features suggestive of leukocytoclastic vasculitis. Diagnosis of acute hemorrhagic edema was made on the basis of clinical examination and skin biopsy. The child responded to steroids.

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Angiolymphoid hyperplasia with eosinophilia (ALHE) is apparently benign, locally proliferating lesion composed of vascular channels with surrounding infiltrate of lymphocytes and eosinophils. We report two cases with long-standing papulonodular lesions on the left ear who presented to us on account of trauma-induced bleeding and cosmetic issues. Peripheral blood eosinophilia was present, and histological findings were consistent with a diagnosis of ALHE. The rarity of this disease in the pediatric age group prompted the present report.

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Hand foot and mouth disease (HFMD) is common viral illness among children characterized by prodromal fever followed by sore throat, rash over hands and feet, and ulcers in mouth. Though rare, patients can develop complications. The treatment of HFMD is usually supportive. It is important for us as a health care professional to be aware about this condition to prevent complications. Good hygienic practices are the most important preventive strategies to stop epidemics. A series of eight cases diagnosed as HFMD on clinical basis are presented here.

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The syndrome is characterized by the association of two classical clinical presentations:“Trichorrhexis invaginata”, reported by Netherton and the“Ichthyosis linearis circumflexa”, described by Comel. Comèl - Netherton syndrome is an autosomal recessive disorder characterized by congenital ichthyosis linearis circumflexa, hair shaft defects, atopy, markedly elevated IgE levels and immune deficiency.1,2,3 It is caused by mutation in Spink5 gene that encodes for protein Latki, which inhibits the enzyme serine proteinase, deficiency of which leads to desquamation of horny layer.4,5 We hereby report this case in a pair of siblings who presented with Ichthyosis linearis circumflexa, trichorrhexis invaginata, pili torti and atopic features. Investigations of skin, hair and blood confirmed the clinical findings. The case is being reported for its rare and classical occurrence.

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Cockayne syndrome (CS) is a rare genodermatosis with autosomal recessive inheritance and around 180 cases have been reported worldwide. It results from mutation in genes ERCC8 and ERCC6 coding for proteins involved in transcription-coupled repair. It is characterized by profound growth retardation, microcephaly, neurodevelopmental impairment, photosensitive skin eruption, premature skin aging, disproportionate large hands, feet and ears, ocular defects, and extensive demyelination. It spans a phenotypic spectrum that includes classic (CS-I), rare severe form with onset from birth (CS-II) and late onset milder form (CS-III). Here, we report an infant with neonatal onset CS-II along with a brief review of the literature.

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We report a rare fatal case of disseminated blastomycosis in an otherwise healthy 12-year-old child. He presented with multiple verrucous, hyperkeratotic suppurating plaques, and multiple sinuses discharging thick pus over the face, chest, and upper and lower limbs for the last 8 months. Clinical diagnosis was confirmed by histopathology which showed thick-walled, clustered refractile yeast cells with broad-based unidirectional budding resembling hyphae. Although he was vigorously treated by intravenous amphotericin-B and itraconazole, the child succumbed to death within 2 weeks of presentation. This case is being reported for its rarity and fatal outcome. Besides, in the present case, widespread cutaneous spread occurred without any other systemic involvement.

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Goltz syndrome, also known as focal dermal hypoplasia, is a rare genodermatosis. It is an X-linked dominant disorder characterized by mesoectodermal dysplasia present with cutaneous, ocular, dental, and skeletal defects. Its hallmark is thinning of the dermis resulting subcutaneous fat herniation. We report a case of a 7-month-old female child with typical clinical features of Goltz syndrome with some unusual findings such as severe abdominal dehiscence, bladder exstrophy, and absence of the fifth metacarpal and distal phalanx.

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Coexistence of two chronic granulomatous diseases in an individual is rare. Here, we present coexistence of lupus vulgaris (LV) and pure neuritic leprosy with incomplete claw hand deformity in an 11-year-old immunocompetent girl, who presented with deformity and ulcer involving right upper limb. Characteristic clinical features and histopathology of biopsy from the ulcer assisted in establishing the diagnosis of LV and leprosy.

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Introduction: Skin diseases are a major health problem in pediatric age group. Skin biopsy and histopathological examination are required in clinically challenging situation to clinch the correct diagnosis. Aims and Objectives: To recognize clinical and histopathological relevance in different pediatric dermatoses and to compare the results. Materials and Methods: A total of 85 skin biopsies were taken over a period of 3 years between August 1, 2012, and July 31, 2015, at our tertiary care hospital and were analyzed by histopathological study. Results: A total of 85 pediatric patients were included in this study. Of these, female were 48 (56.47%). Main diseases diagnosed during the study were bacterial diseases – 20 (23.52%), noninfectious papulosquamous lesions – 13 (15.29%); nevi, vascular tumors and malformations, and viral diseases were 6 (7.05%) each. Conclusion: The histopathologic diagnosis inturn is used as an adjuvant to clinical diagnoses to aid in the management of patients.

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Introduction: Skin disorders are major health problem in the pediatric age group and are associated with significant morbidity. The pattern of pediatric dermatoses has varied in different studies. The majority of dermatoses belonged to infection and infestation group followed by eczematous and hypersensitivity groups. Objective: To study the clinical pattern of infection and infestation in pediatric age group. Materials and Methods: All children 0–12 year of either sex, attending Dermatology Outpatient Department between January 2014 and July 2014 are recruited in this study. A detailed history, complete dermatological examination, along with routine investigations wherever required are recorded in predesigned proforma. Results: The study comprised of 120 patients. A total of 127 cases of infection and infestation were noted. Seven patients were having both. Total of 69.1% infections and 36.7% infestations were noted. Among the various infections and infestations, the most common were bacterial infection 37.5%, fungal infection 15% and viral infection 16.7% and scabies 29.2%, pediculosis 9%. Conclusion: The dermatoses such as infections and infestations in the pediatric age group are common including rural and urban areas.

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